Dr.Muhammad Yusuf,SpSBagian Neurologi PSPD Unimal

DEFINISI

GANGGUAN FUNGSIONAL ATAU ORGANIK

DARI SARAF PERIFER

GANGGUAN INI DAPAT MENGENAI :

SARAF SENSORIK

SARAF MOTORIK

SARAF OTONOM

KOMBINASI

KLASIFIKASI

BANYAK KLASIFIKASI DARI NEUROPATI.

1.MENURUT ONSET SERANGAN:

NEUROPATI AKUT

MIS : POLINEUROPATI IDIOPATIK AKUT

NEUROPATI KRONIK

MIS : BERI BERI

DIABETES MELLITUS

LEPRA

2.MENURUT DERAJATNYA

1.NEUROPATI RINGAN:SENSORIK SAJA

2.NEUROPATI SEDANG :SENSORIK, MOTORIK,

REFLEKS 3.NEUROPATI BERAT :

SENSORIK, MOTORIK,

REFLEKS , ATROFI OTOT

3. MENURUT JUMLAH SARAF YANG TERLIBAT

1. MONONEUROPATI SIMPLEKS:

GANGGUAN PADA SATU SARAF PERIFER SAJA.

2. MONONEUROPATI MULTIPLEKS:

MENGENAI BEBERAPA SARAF TEPI, BIASANYA TIDAK BERDEKATAN DAN TIDAK SIMETRIS.

3. POLINEUROPATI:

BBRP SARAF TEPI, SIMETRIS DAN SERENTAK, BIASANYA PREDOMINAN DI DAERAH DISTAL.

4. MENURUT LETAK LESI

1 AKSONOPATI DISTAL:

GANGGUAN PADA AKSON.

2. MIELINOPATI :

GANGGUAN PADA SELUBUNG MIELIN.

3. NEURONOPATI :

GANGGUAN PADA BADAN SEL SARAF DI CORNU ANTERIOR, MEDULLA SPINALIS ATAU PADA DORSAL ROOT GANGLION.

ETIOLOGI

1. IDIOPATHIC INFLAMMATORY NEUROPATHIES - POLINEUROPATI IDIOPATIK AKUT

(GUILLAIN BARRE SYNDROME)

- CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY

2. METABOLIC AND NUTRITIONAL NEUROPATHIES - DIABETES, HIPOTIROIDI, ACROMEGALI

- UREMIA

- LIVER DISEASES

- VIT B1, OR VIT B12 DEFICIENCY

ETIOLOGI (lanjutan)

3. INFECTIVE AND GRANULOMATOUS NEUROPATHIES:

AIDS, LEPROSY. DIFTERI, SARCOIDOSIS

4. VASCULITIS NEUROPATHIES:

- POLYARTERITIS NODOSA

- RHEUMATOID ARTHRITIS

- SYSTEMIC LUPUS ERYTHEMATOSUS

ETIOLOGI (lanjutan)

5. NEOPLASTIC AND PARAPROTEINEMIC NEUROPATHIES:

- COMPRESSION AND IRITATION BY TUMOR

- PARANEOPLASTIC SYNDROME

- PARAPROTEINEMIAS

- AMYLOIDOSIS

ETIOLOGI (lanjutan)

6. DRUGS INDUCED AND TOXIC NEUROPATHIES

- DAPSON, ISONIAZIDE, PHENYTOIN, PIRIDOKSIN

VINCRISTIN, HIDRALAZINE.

- ALKOHOL

- TOKSIN: ORGANOPHOSPHAT

ARSENIC

LEAD

THALIUM

GOLD

ETIOLOGI (lanjutan)

7. HEREDITARY NEUROPATHIES

- IDIOPATHIC

HEREDITARY MOTOR AND SENSORY NEUROPATHIES

HEREDITARY SENSORY NEUROPATHIES

FAMILIAL AMYLOIDOSIS

- METABOLIC

PORPHYRIA

METACHROMATIC LEUCODYSTROPHY

ABETALIPOPROTEINEMIA

ETIOLOGI

8. ENTRAPMENT NEUROPATHIES

- UPPER LIMBS

MEDIAN NERVE (CARPAL TUNNEL SYNDROME)

ULNAR NERVE

RADIAL NERVE

- LOWER LIMBS

PERONEAL NERVE

FEMORAL NERVE

OBTURATOR NERVE

MOST COMMON DISEASES AFFECTING THE PERIPHERAL NERVE

“DANG THE RAPIST”

Diabetes Trauma Rheumatic (collagen vascular)

Alcohol Hereditary AmyloidNutritional Environmental ParaneoplasticGuillain Barre toxin and drugs Infections

Systemic diseasesTumors

PATOFISIOLOGI

ADA BEBERAPA PROSES PATOLOGI YANG MENGENAI SERABUT SARAF a.l.:

1. DEGENERASI WALLERIAN

TERJADI DEGENERASI AKSON DAN SELUBUNG MIELIN KEARAH DISTAL DARI LESI.

DEGENERASI BISA JUGA KE PROKSIMAL SATU ATAU DUA SEGMEN.

PATOFISIOLOGI

2. DEMIELINISASI SEGMENTAL

TIMBUL BILA TERJADI LESI PADA SEL SCHWANN

PROSES DIMULAI DI DAERAH NODUS RANVIER DAN MELUAS TAK TERATUR MENGENAI SEGMEN-SEGMEN INTERNODUS LAIN.

AKSON DAPAT MENGALAMI DEGENERASI ATAU TIDAK TERGANGGU SAMA SEKALI.

PATOFISIOLOGI

3. DEGENERASI AKSON PRIMER

DISEBUT JUGA DENGAN AKSONOPATI. DEGENERASI AKSON INI BIASANYA DI IKUTI OLEH DEMIELINISASI SEGMENTAL YANG SEKUNDER.

SERING PADA UREMIA, KERACUNAN ALKOHOL, LEPRA, KARSINOMA.

PATOFISIOLOGI

KERUSAKAN SARAF DIBAGI 3 TINGKAT PENTING UNTUK MENENTUKAN PROGNOSE.

1. NEUROPRAKSIA:

- KERUSAKAN PALING RINGAN

- HANYA TERJADI GANGGUAN HANTARAN

- TANPA GANGGUAN KONTINUITAS

- PEMULIHAN TERJADI DALAM BEBERAPA MENIT

SAMPAI BEBERAPA MINGGU

PATOFISIOLOGI

2. AKSONOTMESIS:

- KERUSAKAN PADA AKSON DISERTAI

DEGENERASI

- TANPA KERUSAKAN ENDONEURAL

- REGENERASI KEMUNGKINAN DAPAT

TERJADI DENGAN HASIL YANG BAIK

PATOFISIOLOGI

3. NEUROTMESIS:

- SARAF TERPUTUS TOTAL ATAU

SEBAGIAN

- PENGOBATAN DGN PENYAMBUNGAN

- KEMUNGKINAN PERBAIKAN 50%

GEJALA KLINIK

1. GANGGUAN SENSORIK:

Involvement of sensory axons produces

impairment of sensation with dysesthesias or

paresthesias.

- RASA KAKU, DINGIN, PEDAS

- GATAL DAN KEBAS-KEBAS

- NYERI SEPERTI DITUSUK JARUM

- RASA TERBAKAR

- RASA BERJALAN DI ATAS KAPAS

- RASA TERSANDUNG WAKTU BERJALAN

- RASA TIDAK STABIL

GEJALA KLINIK

2. GANGGUAN MOTORIK:

Involvement of motor axons produces muscle

wasting and weakness followed by atrophy and

fasciculations

- KELEMAHAN BERSIFAT LMN

- SULIT MEMUTAR KUNCI PINTU

- SULIT MEMBUKA KANCING BAJU

- SULIT MEMUTAR TUTUP BOTOL

- FOOT DROP

- WRIST DROP

- GANGGUAN GERAKAN TANGKAS

GEJALA KLINIK

3. GANGUAN REFLEKS TENDON:

The tendon reflexes supplied by the affected nerve

are depressed or absent.

Contoh :

- REFLEKS TENDON BISEPS

- REFLEKS TENDON TRISEPS

- KPR

- APR

GEJALA KLINIK

4. GANGUAN OTONOMIK:

Involvement of axons supplying autonomic

function produces loss of sweating, alteration

in bladder fuction, constipation, and impotence

in male

Contoh : - GANGGUAN GASTROINTESTINAL:

DIARE, KONSTIPASI, DILATASI LAMBUNG, MUAL DAN MUNTAH.

GEJALA KLINIK

GANGGUAN OTONOMIK (lanjutan) : - GANGGUAN KANDUNG KEMIH :

ATONI KANDUNG KEMIH, RESIDU URINE

- IMPOTENSI

- GANGGUAN KARDIOVASKULER:

HIPOTENSI ORTOSTATIK, SINKOP

- GANGGUAN BERKERINGAT

- CARDIO RESPIRATORY ARREST

PREDOMINANTLY MOTOR NEUROPATHIES

Guillain-Barre SyndromeDiphtheric neuropathyDapsone-induced neuropathyPorphyria and multifocal motor

neuropathy

PREDOMINANTLY SENSORY NEUROPATHIES

Drug toxicity : pyridoxine, doxorubicine

Autoimmune : paraneoplastic, Sjogren syndrome, etc.

Infectious : diphtheria, HIVDeficiency : vit. EInherited : abetalipoproteninemia.

DIAGNOSA

1. GEJALA KLINIK

2. LABORATORIUM

3. FOTO THORAKS

4. PUNKSI LUMBAL

5. EKG

6. BIOPSI : paling sering n. suralis atau n. cutaneus radialis

7. ELEKTROFISIOLOGI: EMG

NCV

ELEKTRO MIOGRAFI

ELEKTRODA DITUSUKKAN KEDALAM SUATU OTOT SKELET UNTUK MEMPELAJARI PERUBAHAN POTENSIAL LISTRIKNYA.

INDIKASI:

GANGGUAN LOWER MOTOR NEURON, YANG LESINYA DI:

1. KORNU ANTERIOR

2. RADIKS

3. PLEKSUS

4. SARAF PERIFER

5. NEUROMUSCULAR JUNCTION

6. OTOT

MANFAAT EMG

MEMBANTU DIAGNOSA SECARA DINI MENENTUKAN LETAK LESI MEMBEDAKAN LESI MIOGEN ATAU NEUROGEN MENENTUKAN LESI PARSIAL ATAU TOTAL MEMBEDAKAN SENSORIK ATAU MOTORIK EVALUASI PENGOBATAN MEMBANTU MENENTUKAN PROGNOSE

NERVE CONDUCTION VELOCITY( NCV)

NCV ATAU KHS

NILAI NORMAL :

N. ULNARIS = 47 - 72 m / s

N. MEDIANUS = 46 - 72 m / s

N. PERONEUS = 42 - 63 m / s

N. TIBIALIS = 40 - 67 m / sDISTAL LATENCY ( DL )

NILAI NORMAL N. MEDIANUS

2,7 + 0,3 m/s

MANFAAT PENGUKURAN KHS

MENGIKUTI PERJALANAN PENYAKITMENGEVALUASI EFEK PENGOBATANMENENTUKAN PROGNOSE, APAKAH

MASIH MUNGKIN DIPEROLEH PERBAIKAN LAGI.

EMG DAN KHS PADA NEUROPATI

DIJUMPAI PENURUNAN KHS.PEMANJANGAN DISTAL LATENCYPENURUNAN AMPLITUDO GELOMBANG MDURASI YANG MEMANJANGPOTENSIAL POLIFASIKFIBRILASI

NEUROPATI DIABETIK

PREVALENSI : 10 - 20 % (SIMTOMATIK)KHS 80 % ABNORMALKLINIS DAPAT MENGENAI:

SENSORIK

MOTORIK

OTONOMIK

KOMBINASI

PATOGENESE NEUROPATI DIABETIK

The etiology is uncertain.

4 hypothesis (not necessarily exclusive) :

1. Hyperglycemia-polyol-myoinositol hypothesis.

2. Microvascular hypothesis

3. Structural changes at the node of Ranvier.

4. Vasculitic neuropathy.

1. Hyperglycemia-polyol-myoinositol

hypothesis

Normal : glucose hexokinase glucose-6-phosphate Krebs cycle.

Hyperglycemia saturates hexokinase activity glucose shunted to polyol pathway production of sorbitol assoc w/ a decrease in intracelluler myoinositol defective Na/K ATPase activity defect axon transport slowing NCV

2. Microvascular hypothesis

DM : ** thickening of capillary basement membrane

** increase in the size and number of capillary endothelial cells

Microangiopathy increase number of closed capillaries in peripheral nerves progressive hypoxia secondary changes in axons and Schwann cells

3. Structural changes at the node of Ranvier

Na/K ATPase defiency increase intra-axonal Na and nodal axonal swelling detachment of myelin myelin retraction from the nodal area slowing of axonal conduction.

Exposure of paranodal K channels leakage of K impairment of axonal conduction.

Impairment of axonal transport gradual dying back of axons starting at the distal axons and progressing proximally.

4. Vasculitic neuropathy

Some cases of NIDDM and proximal diabetic have a inflammatory vasculopathy with perivascular collections of lymphocytes and axonal neuropathy

PAINFUL DIABETIC NEUROPATHY

cranial nerve neuropathyAcute thoracoabdominal neuropathyAcute distal sensory neuropathyAcute lumbar radiculoplexopathyChronic distal small-fiber neuropathy

Terapi

Intensive diabetic therapyMaintain ideal body weightAdjuvant analgetics :

TCA antidepressantscarbamazepinegabapentinintravenous lidocaine, etc

CARPAL TUNNEL SYNDROME

CHARACTERIZED BY :

FLUCTUATING NUMBNESS, PARESTHESIA AND PAIN IN THE HAND DUE TO COMPRESSION OF THE MEDIAN NERVE AT THE WRIST.

80% in WOMEN, A COMMON TEMPORARY PHENOMENON DURING PREGNANCY

PRESSURE TO THE NERVE WHEN PASSING BENEATH THE FLEXOR RETINACULUM OBSTRUCTION OF VENOUS CIRCULATION AND EDEMA ISCHEMIA INCREASING PRESSURE ON THE NERVE ISCHEMIC ATROPHY OF NERVE FIBERS

Etiologi

1. Hereditary : HMSN type III2. Traumatic : dislocation, fracture, hematoma, wrist

sprain3. Infection : tenosynovitis, tbc, sarcoidosis4. Metabolic : amyloidosis, gout5. Endocrine : acromegaly, DM, hypothyroidism,

pregnancy6. Neoplastic : ganglion cysts, lipoma , myeloma7. Collagen vascular diseases : RA, polymyalgia

rheumatica, SLE8. Degenerative disease : OA9. Iatrogenic : radial artery puncture, shunt for dialysis,

anticoagulant therapy

Gejala Klinis

The earliest symptoms : numbness and paresthesias in the sensory distribution of the median nerve in the hand (thumb, index, middle and lateral half of the ring finger)

Later on : pain, worst at nightLate : inability to screw bottle caps or grip

properly

Terapi

Identified causes should be treatedCorticosteroid injection around the median

nerve in the carpal tunnel.Surgical division of the transverse

ligament (flexor retinaculum)Endoscopic carpal tunnel release

GUILLAIN - BARRE SYNDROME (GBS)

ACUTE INFLAMMATORY POST INFECTIOUS

POLYNEUROPATHY

- INSIDEN: 2 PER 100.000 POPULASI PERTAHUN

- 1-3 MINGGU SETELAH INFEKSI :

VIRUS

BAKTERI

IMUNISASI

G B S (lanjutan)

- INFLAMASI TERHADAP SERABUT SARAF MERUPAKAN RESPON AUTOIMMUN BAIK MELALUI REAKSI ANTIBODI MAUPUN CELL MEDIATED RESPONSE

- TERJADI DEMIELINASI SEGMENTAL DISERTAI DENGAN KERUSAKAN AKSON BILA PROSESNYA BERAT

- DIJUMPAI INFILTRASI LIMFOSIT PERIVASKULER PADA SARAF PERIFER DAN NERVE ROOTS

- LIMFOSIT DAN MAKROFAG MENGHASILKAN SITOTOKSIN YANG MERUSAK MIELIN

G B S (lanjutan)

- KELUMPUHAN KEEMPAT ANGGOTA GERAK

- UMUMNYA DIMULAI DARI TUNGKAI, MELUAS KEATAS, LENGAN, OTOT LEHER DAN WAJAH KADANG-KADANG OTOT MENELAN

- SEBAGIAN BESAR KASUS MENGELUH PARASTESI PADA EKSTREMITAS INFERIOR

- GANGGUAN OTONOMIK DIJUMPAI PADA

25% KASUS

- PADA LP DIJUMPAI DISOSIASI SITOALBUMIN

G B S (lanjutan)

KRITERIA DIAGNOSTIK

DIJUMPAINYA 5 DARI 6 KRITERIA INI :

1. DIFFUSE FLACCID PARALYSIS

2. GANGGUAN SENSORIK < GANGGUAN MOTORIK

3. REMISI SEMPURNA DALAM 6 BULAN

4. PENINGKATAN PROTEIN PADA CSF DLM 2 MGG

5. DEMAM (-) ATAU SUHU SEDIKIT MENINGGI

6. LEUKOSIT NORMAL HANYA KEMUNGKINAN LED

SEDIKIT MENINGGI

PREDICTORS OF SEVERE DISEASE AND POORER OUTCOME

Old ageRapid onset of severe tetraparesisNeed for early artificial ventilationSeverely decreased compound

muscle action potential (<20% normal)

Acute motor-sensory axonal form of the disease

TERAPI

1. PLASMAPHARESIS

2. IMMUNOGLOBULIN IV 0,4 gr/kg BB

SELAMA 5 HARI

3. PERAWATAN UMUM

4. FISIOTERAPI

5. PERAWATAN DI ICU BILA TERJADI

GAGAL NAFAS